Clinical manifestations of essential thrombocythemia in young adults.
Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by isolated overproduction of platelets, thrombohemorrhagic complications, and a median age of 50-60. When it occurs in younger patients, the incidence of complications has been reported to be quite low, with a good long-term prognosis. We report a retrospective review of 13 patients with ET between the ages of 22 and 35 in which 11 were symptomatic at diagnosis, with only one remaining asymptomatic during follow-up. Three patients presented with potentially life-threatening complications (two myocardial infarctions, one stroke), although no deaths were observed. The majority of the nonlife-threatening complications were vaso-occlusive in nature, including erythromelalgia and transient neurologic symptoms. We conclude that ET in young adults is not always a benign disease and that potentially life-threatening complications are not rare. The optimum approach to treatment in this or any other age group remains uncertain.
Millard
F E
FE
Department of Internal Medicine, Division of Hematology/Oncology, Naval Hospital San Diego, California 92134-5000.
Hunter
C S
CS
Anderson
M
M
Edelman
M J
MJ
Kosty
M P
MP
Luiken
G A
GA
Marino
G G
GG
D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013920:Thrombocythemia, Essential / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy